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Lymphomatoid papulosis: clinicopathological comparative study with pityriasis lichenoides et varioliformis acuta.

We have compared the clinical and histopathological features of 6 patients with lymphomatoid papulosis (LP) and 14 patients with pityriasis lichenoides et varioliformis acuta (PLEVA). There were some differences between the clinical features in the two diseases, including the size and appearance of skin lesions and the duration of the course of disease. Ki-1 Ag positive, large, atypical, lymphoid cells were always seen in lymphomatoid papulosis; none of lymphoid cells of pityriasis lichenoides et varioliformis acuta demonstrated this antigen. We conclude that lymphomatoid papulosis and PLEVA, although sharing some common features, should be considered to be different clinical and immunopathological entities.

Adolescent↗

Pityriasis rosea-like eruption due to pneumococcal vaccine in a child with nephrotic syndrome.

A pityriasis rosea-like eruption can occur as a consequence of treatments with gold compounds and captopril. It has rarely been reported to have an association with vaccinations such as smallpox, BCG, hepatitis B, and diphtheria toxoid. It has not previously been documented to develop after pneumococcal vaccination. We report a case of pityriasis rosea-like eruption that developed following pneumococcal vaccination in a child with nephrotic syndrome.

Biopsy, Needle↗

Ultrastructural studies of epidermal lesions in pityriasis lichenoides chronica: occurrence of tubular aggregates and intracytoplasmic desmosomes.

Very early skin lesions from four patients with pityriasis lichenoides chronica were examined by electron microscopy. Aggregates of tubular structures, which resembled those reported in endothelium in lupus erythematosus and are currently being observed in an increasing variety of conditions, were noticed in the epidermal cells of all patients. These cells also showed intracytoplasmic desmosomes, and isolated mitochondria and myelin-like figures in intercellular spaces, in addition to the occurrence of vacuoles and lysosomes in the cytoplasm, dilatation of endoplasmic reticulum and alteration of mitochondria. Occasionally, epidermal cells were seen to be individually filled with fibrils. These ultrastructural findings seem to indicate that in pityriasis lichenoides chronica some injury to epidermal cells occurs at the early stage of pathological processes, as suggested by light microscope studies.

Adult↗

Pityriasis lichenoides--an immune complex disease.

Circulating immune complexes have been detected in patients with pityriasis lichenoides during disease activity when IgM and C3 have been observed in dermal vessels on direct immunofluorescence of fresh lesions. This implies that pityriasis lichenoides is an immune complex disorder and that deposited complexes play a part in the pathogenesis of the condition. There is a characteristic pattern of immunofluorescence which may be a diagnostic aid.

Adolescent↗

An immunofluorescence study of pityriasis lichenoides.

In a study of 27 patients with pityriasis lichenoides IgM and C3 have been observed on direct immunofluorescence of fresh lesions. Other immunoglobulins and complement components were not observed. Immunofluorescence was seen in 31 (72%) of 43 fresh lesions. It occurred in the walls of superficial dermal vessels and along the dermal-epidermal junction. This pattern of immunofluorescence appears to be characteristic of the disease. Uninvolved skin showed the immunofluorescence less frequently and old scaly lesions none. The concept that pityriasis lichenoides is an immune complex disorder is discussed.

Complement C3↗

Pityriasis rubra pilaris and and retinol-binding protein.

Serum levels of retinol-binding protein (the specific carrier of vitamin A) were measured in eleven patients with pityriasis rubra pilaris and in some of their close relatives. The level of retinol-binding protein was markedly reduced in every patient, and in some of the relatives. It is postulated that defective synthesis of retinol-binding protein is a biochemical marker for pityriasis rubra pilaris, probably transmitted as a Mendelian dominant.

Adolescent↗

Dyskeratotic degeneration of epidermal cells in pityriasis rosea: light and electron microscopic studies.

We studied biopsies from twenty-nine patients with pityriasis rosea. All specimens showed features of subacute or chronic dermatitis as previously reported, but sixteen cases (55%) had dyskeratotic cells within the epidermis. Ultrastructurally, these cells contained an aggregation of tonofilaments, many vacuoles and intracytoplasmic desmosomes. Previously, only non-specific histological changes had been described in pityriasis rosea, but the presence of dyskeratotic cells seems to be a significant feature.

Adolescent↗

HLA-DR antigen expression on the keratinocyte surface in dermatoses characterized by lymphocytic exocytosis (e.g. pityriasis rosea).

We have investigated the immunoperoxidase staining pattern of the epidermis in several dermatoses characterized by exocytosis of mononuclear cells into the epidermis. We found that HLA-DR antigens showed an intercellular distribution in localized areas of the epidermis in nine of ten cases of pityriasis rosea, and in all four cases of spontaneously regressing flat warts, two cases of pityriasis lichenoides chronica, two of Schamberg's disease, and one case of lichen striatus. Lichen planus and mycosis fungoides cases were used as positive controls. OKT6 antigen was recognized only on the dendritic cells of the epidermis in all these cases. Judging from the distribution of Langerhans cells, the epidermal intercellular HLA-DR antigen seems to be expressed on the keratinocytes in such diseases, and this feature was confirmed by immunoelectron microscopy. These findings support the hypothesis that the expression of HLA-DR antigen on keratinocytes in these dermatoses is linked to cellular immune reactions involving the epidermis.

Cell Membrane↗

Juvenile pityriasis rubra pilaris associated with hypogammaglobulinaemia and furunculosis.

We report a case of follicular keratosis with inflammatory changes, consistent with a diagnosis of atypical juvenile pityriasis rubra pilaris. An unusual feature was the occurrence of severe Staphylococcus aureus folliculitis and furunculosis, a phenomenon rarely encountered in pityriasis rubra pilaris and the other follicular keratoses. Standard antibiotic and antiseptic treatment for chronic S. aureus infection was ineffective. The patient was subsequently found to have hypogammaglobulinaemia, and treatment with human polyvalent immunoglobulin infusions was successful in eradicating the sepsis. It is therefore probable that the hypogammaglobulinaemia played a pathogenic role in the development of cutaneous sepsis.

Adult↗

Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) represents a fulminant and potentially lethal variant of pityriasis lichenoides. Only 24 cases have been described so far. We report a 9-year-old boy who initially presented with classical pityriasis lichenoides et varioliformis acuta (PLEVA) following a mild enteritis. Three weeks later, his skin lesions started to ulcerate progressively, involving > 90% of his body surface, accompanied by high fever, normal C-reactive protein, but highly elevated serum levels of tumour necrosis factor (TNF)-alpha. Methotrexate 10 mg m(-2) weekly was required to halt disease progression, while oral steroids (initial dose 2.8 mg kg(-1) daily) alone proved insufficient. Sequential histology revealed progressively dense perivascular and intramural lymphocytic inflammation as well as keratinocyte necrosis. Our case demonstrates the clinical and histological continuum between 'classical' PLEVA and FUMHD and points to the potentially pathogenic significance of TNF-alpha. We hypothesize that in future cases, treatment with TNF-alpha antagonists might represent a reasonable alternative to high-dose immunosuppressive therapy.

Administration, Oral↗

Pityriasis rotunda in a Caucasian woman from the Mediterranean area.

Pityriasis rotunda (PR) has been known to affect, almost exclusively, Japanese and South African Bantu, often in association with various systemic diseases. However, the occurrence of PR in Caucasian patients is extremely rare, and has been previously described in only three reports. We hereby report a case of an apparently healthy Caucasian woman from Israel having clinical and histological features characteristic of PR. Pityriasis rotunda (PR) is a rare disorder of keratinization characterized by strikingly well demarcated, perfectly round scaly brownish patches of variable number and diameter located mainly on the trunk and extremities. The exact place of this disorder within the nosology of the cutaneous dermatoses is not yet fully established. However, most authors regard it as a special circumscribed form of acquired ichthyosis with a histological resemblance to ichthyosis vulgaris. For more than half a century following the first description of this disorder in 1906 cases reported were almost exclusively from the Far East, particularly from Japan, or in South African Negroids, with a few cases of West Indian Negroids, usually in association with various debilitating diseases. The first report of a Caucasian patient appeared in the literature in 1966 and since then there have been only two additional reports of PR in Caucasian patients.

Adult↗

Pityriasis rosea in the Sudan.

Pityriasis rosea presents in the Sudan with the same picture as it does in other countries of Africa. The seasonal incidence is highest in the cold, dry months. The erythematous or rosea picture seen in whites is never encountered in blacks. The term pityriasis rosea may not be descriptive of the disease as it presents in black and brown skins.

Adolescent↗

Light and electron microscopic studies of pityriasis rosea.

Light and electron microscopic studies of biopsy specimens taken from five patients with pityriasis rosea revealed the most conspicuous finding to be the presence of dyskeratotic cells within the epidermis. Ultrastructurally, these cells contained an aggregation of tonofilaments, intracytoplasmic desmosomes, many vacuoles, and viral-like particles. It is assumed that pityriasis rosea is caused by a virus to which these changes are related.

Adolescent↗

Familial pityriasis rotunda.

Pityriasis rotunda is a rare dermatosis characterized by circular, dusty scaling, dyschromic patches, quite asymptomatic and mostly described in Japanese and blacks. The authors have seen three cases of pityriasis rotunda in a Sardinian family that are to be added to two other similar reports. The patients were all in good health. An interesting feature was the association with favism. On inquiry it was discovered that many more members of the family were affected by either or both pathologies. The authors believe this condition to be a form of minor acquired ichthyosis of which Sardinia could be an ethnic center.

Adult↗

Is human herpesvirus 7 the causative agent of pityriasis rosea?--A critical review.

BACKGROUND: Conflicting results on the association of pityriasis rosea and human herpesvirus 7 infection have been reported by different investigators. AIM: To review the level of evidence for such an association. METHODS: Medline was searched with unlimited data entry and 13 reports were retrieved. The data were analyzed for a causative association according to the criteria of Fredericks and Relman, which take into consideration latent infection and the reactivation of viruses characteristic of herpesviruses, and the roles of sequence-based detection methods. RESULTS: None of the criteria was substantiated by the findings of most investigators. Factors leading to the discrepancies of the results were discussed. CONCLUSION: There is currently insufficient evidence that human herpesvirus 7 infection is causally related to pityriasis rosea.

DNA, Viral↗

Investigation of herpes simplex virus DNA in pityriasis rosea by polymerase chain reaction.

BACKGROUND: Pityriasis rosea (PR) is an acute, inflammatory disease of unknown cause. Clinical and experimental findings indicate an infectious etiology of PR. Our purpose is to examine the skin lesions and blood samples of PR patients by polymerase chain reaction (PCR) for the presence of HSV type 1 and 2 DNA. METHODS: The lesional skin biopsies from 10 patients and blood samples from two randomized patients with clinically and histologically confirmed pityriasis rosea were examined by PCR. RESULTS: No HSV 1 and HSV 2 DNA was detected in the lesional biopsy and blood samples. CONCLUSIONS: We could not identify a relationship between HSV 1, HSV 2 and PR.

Adult↗

Effect on quality of life in patients with pityriasis rosea: is it associated with rash severity?

BACKGROUND: It is unknown how the quality of life (QOL) is affected in patients with pityriasis rosea (PR), and whether it is related to rash severity. METHODS: We constructed a valid and reliable Cantonese version of the Dermatology Life Quality Index (DLQI). We recruited patients with PR, with atopic dermatitis and with acne vulgaris, and controls of the same sex and comparable age. We applied the DLQI and the Pityriasis Rosea Severity Score (PRSS) to the patients with PR, the DLQI and the SCORing Atopic Dermatitis Index (SCORAD) to the controls with atopic dermatitis, and the DLQI and the Leeds Acne Grading System (LAGS) to the controls with acne vulgaris. RESULTS: Total DLQI scores of the 22 patients with PR (mean: 6.36, SD: 5.79) were significantly lower than those of the 22 controls with atopic dermatitis (mean: 12.00, SD: 5.38) (P = 0.021), but were insignificantly different from those of the 22 controls with acne vulgaris (mean: 6.86, SD: 4.53) (P = 0.57). Correlation between the total DLQI and PRSS scores was weak (gamma(s) = +0.19) and insignificant (P = 0.40). All six DLQI parameters were insignificantly correlated with the PRSS scores. In contrast, the total DLQI scores and most of the DLQI parameters were strongly correlated with the rash severity scores for the control subjects. Significantly more patients with PR have concerns regarding disease etiology and infectivity. CONCLUSIONS: The QOL of the patients with PR was significantly less affected than that for the patients with atopic dermatitis, but was insignificantly different from the patients with acne vulgaris. Unlike atopic dermatitis and acne vulgaris, the effects on the QOL in PR are insignificantly correlated with rash severity. These results bear important implications on clinical management.

Acne Vulgaris↗

Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.

We report life-threatening febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta in an 8-year-old girl. Hemorrhagic-crusted papules and plaques covered over 90% of the patient's body, leaving her susceptible to Pseudomonas aeruginosa and Staphylococcus epidermidis bacteremia as well as Candida parapsilosis fungemia. Sepsis delayed definitive treatment of the underlying cutaneous disease for 2 weeks. Combined therapy with methotrexate and cyclosporin caused remission of the process. Although immunohistochemistry revealed CD-30 positive cells, suggesting the diagnosis of lymphomatoid papulosis, the histopathology was most compatible with pityriasis lichenoides et varioliformis acuta. A partial loss of CD2 and CD5 in the predominant CD3 T-cell lymphocytes suggested a clonal proliferation. Elevated soluble interleukin-2 receptor levels reflected marked T-cell activation, and the downward trend of the levels during treatment coincided with clinical regression of this inflammatory dermatosis.

Anti-Bacterial Agents↗